Amantadine can Ameliorate Lower Urinary Tract Dysfunction and Nocturnal Polyuria in Patients with Parkinson Disease and Vascular Parkinsonism

Background:Amantadine is a drug used for patients with Parkinson\u27s disease (PD) and vascular parkinsonism (VP). These patients often have lower urinary tract symptoms (LUTS) and nocturnal polyuria (NP). Thus, we investigated the effect of amantadine on these in parkinsonian patients.Methods:Twenty-two patients with LUTS, including 13 with PD and nine with VP, were recruited. We performed a urinary questionnaire, frequency-volume chart, and residual urine (RU) measurement before and after daily administration of 150 mg and 300 mg amantadine.Results:Before amantadine administration, mean daytime urinary frequency was 9.07(standard error [SE], 0.64), nighttime urinary frequency 2.89 (0.24), urinary urgency per week 24.2 (6.69), urge incontinence per month 15.1( 9.94), urine volume per void 145.6( 12.6) mL, and residual urine volume 12.5( 6.30) mL. After daily 150 mg amantadine administration, mean daytime and nighttime urinary frequency, urinary urgency, and urge incontinence decreased to 6.9( 0.42), 1.97( 0.21), 13.0( 3.58), and 14.2( 10.2), respectively, and urine volume per void increased to 174.1( 11.3) mL. NP( N=8) was ameliorated in six patients. No patient had side effects. After daily 300 mg amantadine administration( N=8), mean daytime and nighttimeurinary frequency, urinary urgency, and urge incontinence decreased to 6.90 (0.33), 1.69 (0.10), 5.88 (1.61), and 2.31 (0.61), respectively, and urine volume per void increased to180.2 (15.0) mL. NP (N=4) was ameliorated in two patients. One patient developed hallucination, and two patients developed flashing sensation.Conclusion:Amantadine has beneficial effects on LUTS and NP in patients with VP and PD

パーキンソン カンレン シッカン ニオケル ケイズガイ チョウオンパ ケンサ ニヨル チュウノウ コクシツ ノ コウキド ヘンカ ノ ケントウ

目 的:パーキンソン病 (Parkinson disease:PD),多系統萎縮症 (multiple system atrophy:MSA),進行性核上性麻痺 (progressive supranuclear palsy:PSP) の患者において経頭蓋超音波検査 (transcranial sonography:TCS) よる中脳黒質の高輝度変化を検討した.方 法:パーキンソン関連疾患連続110 例 (PD 86 例,MSA 12 例,PSP 12 例) と健常者34 例に対しTCSを施行した.中脳黒質を観察しえたPD 47 例,MSA 10 例,PSP 6 例,健常者32 例を解析対象として中脳黒質高輝度所見を評価した.定性評価は高輝度の程度によって視察的にI:none or faint,II:equivocal,III:definite,IV:marked の4 段階に分類した.定量評価は中脳黒質で高輝度変化の面積が0.20 cm2 以上のとき,病的な黒質高輝度変化と定義した.結 果:定性評価では,高輝度範囲が視察的に病的と判定されるIII+IVの割合は,PD 72.4%,MSA 10.0%,PSP 66.7%,健常者3.1%であった.定量評価では,PD 63.8%,MSA 20.0%,PSP 66.7%,健常者9.4%で病的な高輝度変化をみとめた.PD,PSP で病的な高輝度変化の割合が多かった.PSP をPSP-parkinsonism( PSPP)とRichardson\u27s syndrome の2 群に分けた場合,前者では病的な高輝度変化を3 例中3 例 (100%), 後者では3 例中1 例( 33.3%) に認められ,PSP-P で割合が高かった.MSA では10 例中2 例( 20%) に病的な高輝度を認め,いずれもパーキンソン病型の多系統萎縮症であった.結 論:パーキンソン関連疾患における病的な中脳黒質高輝度変化は,疾患特異性というよりも,パーキンソニズムの症候と関連し,ドパミン神経細胞の脆弱性を示す所見と推察された.Objective:We investigated substantia nigra (SN) hyperechogenicitydetermined by transcranial sonography(TCS) to detect abnormalities, and compare findings withthose from Parkinson disease (PD), multiple system atrophy(MSA), progressive supranuclear palsy (PSP) or controlsubjects.Method:In this study, echogenicity of SN was examinedin consecutive 110 parkisonian disorders patients with PD86, MSA12, PSP 11, and 34 control subjects. A sufficientbone window for TCS was available in 47 of 86( 71.2%) inthe PD group, 10 of 12( 86.3 %) in the MSA group, 6 of 11(54.5%) in the PSP group and 32 of 34( 94.1%) in the controlgroup. SN hyperechogenicity was scored using a fourpointscale as follows:I=none or faint, II=equivocal, III=definite, IV=marked. In accordance with previously reportedcut-off values, areas of echogenicity £ 0.19 cm2 wereclassified as normal and areas of echogenicity £ 0.20 cm2were classified as pathological SN hyperechogenicity.Results:The frequency of SN hyperechogenicity, assessedas III and IV scales, was significantly increased in PDpatients, and observed in 72 . 4 % of assessable SN(34/47);qui-squire;p=0.001, vs. controls). The meansize of the SN hyperechogenic area in the PD group, MSAgroup and PSP group was 0.26 cm2±0.13, 0.11 cm2±0.11and 0.23 cm2±0.04, respectively, compared with 0.07 cm2±0.06 in the control group.We have identified two clinical phenotypes, such as Richardson\u27ssyndrome (RS) and PSP-parkinsonism (PSP-P).All of three PSP-P (100%) patients showed a pathologicalSN hyperechogenicity.Conclusion:SN hyperechogenicity was associated with asymptom of parkinsonism rather than disease specificity,and suggested a vulnerability marker of the dopaminergicneuron

シンコウセイ カク ジョウセイ マヒ オ ヘイハツ シタ ネマリン ミオパチー ノ セイジン レイ

症例は66 歳の男性.歩行障害を主訴に来院.軽度の認知症と下方優位の垂直性眼球運動障害,下肢の近位筋の筋力低下と姿勢の不安定さ,易転倒性があった.頭部Magnetic resonance imaging( MRI) でhummingbird sign と第3 脳室の拡大,脳血流シンチグラフィで前頭葉の血流低下があり,進行性核上性麻痺(ProgressiveSupranuclear Palsy;PSP) と診断した.また,血清筋原性酵素の上昇があり,Gomori トリクローム変法染色の筋病理所見でネマリン小体が認められネマリンミオパチーと診断した.進行性核上性麻痺を併発したネマリンミオパチーの報告は過去になく,臨床的特徴を検討した.A 66-year-old man who came to the hospital due to gaitdisturbance showed mild dementia, vertical ocular motilitydisorder, loss of muscle strength of proximal muscle of thelower limbs and unstable posture, and a tendency to fall.Brain magnetic resonance imaging (MRI) revealed hummingbird sign and enlargement of the third ventricle. Cerebralblood flow scintigraphy revealed reduced cerebralblood flow in the frontal lobe and frontal release signs, andprogressive supranuclear palsy (PSP) was diagnosed. Serummyogenic enzyme levels were elevated, and nemalinebody was observed in muscle biopsy specimens. A diagnosisof nemaline myopathy was made, and clinical characteristicswere examined. This is the first report of nemalinemyopathy complicated with PSP

アテローム ケッセンセイ コウソク ノ ソウキ チリョウ ワ ヨゴ カイゼン ニ ユウコウ デ アルカ? : ノウソッチュウ キュウセイキ カンジャ データベース オ モチイタ ケントウ

脳梗塞の早期治療は重要だが,超急性期のtissue plasminogen activator (t-PA)の他に,早期治療の有効性は証明されていない.われわれは,t-PA適応のないアテローム血栓性梗塞の早期治療の有効性について検討した.対象はJSSRS脳卒中急性期患者データベースに登録された127例のアテローム血栓性梗塞で,発症から当院到着までを発症一来院時間とし,発症3～6時間の早期来院,6時間以降の非早期来院に分類し,退院時のmodified Rankin Scale (mRS)およびNIHSS改善度を求めた.退院時mRSに差はなかったが,NIHSS改善度はそれぞれ1.9±3.5,1.6±6.4であり,早期来院で有意に改善した.近年,"Brain Attack"の重要性が唱えられているが,t-PA適応に外れた場合でも早期に来院,治療をすることの重要性の一部を裏付けたものと考えられた.Early treatment of cerebral infarction is important, however, the effect of such treatment has not been established yet except for hyperacute treatment using tissue plasminogen activator (t-PA). We investigated the outcome of early treatment at the hospital for stroke care. The study subjects were 127 patients based on a Japanese Standard Stroke Registry Study (JSSRS) database with atherothrombotic infarction admitted 3 hour later after onset. Patients were divided into two groups based on the time from onset of clinical features of stroke to hospital admission; the early visit group (3-6 hr) and late visit group (>6 hr). All patients were assessed by modified Rankin Scale (mRS) and NIH Stroke Score (NIHSS). There were no significant differences of mRS at discharge. However, the degree of improvement of NIHSS score at discharge were 1.9±3.5 and 1.6±6.4, respectively. Patients of the early visit group showed significantly better improvement compared with the late visit group. At special hospital for stroke care, patients with onset-visit period of <6 hr showed significantly better improvement at discharge. Since the importance of the concept of "Brain Attack" was argued recently, our results confirm the importance of early admission and delivery of intensive care on prognosis of stroke patients

Intravenous immunoglobulin treatment for mild Guillain-Barré syndrome. An international observational study

Objective: To compare the disease course in patients with mild Guillain-Barré syndrome (GBS) who were treated with intravenous immunoglobulin (IVIg) or supportive care only. Methods: We selected patients from the prospective observational International GBS Outcome Study (IGOS) who were able to walk independently at study entry (mild GBS), treated with one IVIg course or supportive care. The primary endpoint was the GBS disability score four weeks after study entry, assessed by multivariable ordinal regression analysis. Results: Of 188 eligible patients, 148 (79%) were treated with IVIg and 40 (21%) with supportive care. The IVIg group was more disabled at baseline. IVIg treatment was not associated with lower GBS disability scores at 4 weeks (adjusted OR (aOR) 1.62, 95% CI 0.63 to 4.13). Nearly all secondary endpoints showed no benefit from IVIg, although the time to regain full muscle strength was shorter (28 vs 56 days, p=0.03) and reported pain at 26 weeks was lower (n=26/121, 22% vs n=12/30, 40%, p=0.04) in the IVIg treated patients. In the subanalysis with persistent mild GBS in the first 2 weeks, the aOR for a lower GBS disability score at 4 weeks was 2.32 (95% CI 0.76 to 7.13). At 1 year, 40% of all patients had residual symptoms. Conclusion: In patients with mild GBS, one course of IVIg did not improve the overall disease course. The certainty of this conclusion is limited by confounding factors, selection bias and wide confidence limits. Residual symptoms were often present after one year, indicating the need for better treatments in mild GBS

Second IVIg course in Guillain-Barré syndrome with poor prognosis. The non-randomised ISID study

Objective To compare disease course in patients with Guillain-Barré syndrome (GBS) with a poor prognosis who were treated with one or with two intravenous immunoglobulin (IVIg) courses. Methods From the International GBS Outcome Study, we selected patients whose modified Erasmus GBS Outcome Score at week 1 predicted a poor prognosis. We compared those treated with one IVIg course to those treated with two IVIg courses. The primary endpoint, the GBS disability scale at 4 weeks, was assessed with multivariable ordinal regression. Results Of 237 eligible patients, 199 patients received a single IVIg course. Twenty patients received an α early' second IVIg course (1-2 weeks after start of the first IVIg course) and 18 patients a α late' second IVIg course (2-4 weeks after start of IVIg). At baseline and 1 week, those receiving two IVIg courses were more disabled than those receiving one course. Compared with the one course group, the adjusted OR for a better GBS disability score at 4 weeks was 0.70 (95%CI 0.16 to 3.04) for the early group and 0.66 (95%CI 0.18 to 2.50) for the late group. The secondary endpoints were not in favour of a second IVIg course. Conclusions This observational study did not show better outcomes after a second IVIg course in GBS with poor prognosis. The study was limited by small numbers and baseline imbalances. Lack of improvement was likely an incentive to start a second IVIg course. A prospective randomised trial is needed to evaluate whether a second IVIg course improves outcome in GBS